Computed tomography-guided central venous catheter placement in a patient with superior vena cava and inferior vena cava occlusion.

An 18-year-old man with a gastrointestinal hypomotility syndrome required lifelong parenteral nutrition. Both the superior and inferior vena cava were occluded. Computed tomography guidance was used to place a long-term central venous catheter via a large tributary to the azygos vein.

Delayed primary repair of esophageal atresia with tracheoesophageal fistula: is it worth the wait?

OBJECTIVE: To characterize a successful approach to the management of infants with long-gap esophageal atresia (EA) with tracheoesophageal fistula (TEF), significant prematurity with respiratory distress syndrome (RDS), or both, so as to preserve the native esophagus. DESIGN: A review of the medical records and office charts of a cohort of patients with EA and TEF. SETTING: A tertiary care children's hospital affiliated with a major university. PATIENTS: A total of 118 children with EA and TEF admitted from February 1986 through December 1996. All of the patients diagnosed as having EA and TEF during this period were included. INTERVENTION: Of the 118 infants, 88 received primary repair of EA and TEF within 48 hours of birth. An additional 23 children had the TEF divided and a gastrostomy placed secondary to (1) severe RDS and prematurity (n = 6), (2) long-gap EA (gap length > 4 cm or the upper pouch above the thoracic inlet (n = 10), or (3) associated cardiac defects (n = 7). Delayed primary EA repair was done when the RDS resolved or the gap length was 2 cm or less. MAIN OUTCOME MEASURES: Successful anastomosis of native esophagus. Comparison of incidence of gastroesophageal reflux, anastomotic complications, or survival between groups undergoing primary or delayed repair. RESULTS: Primary EA was accomplished in 88 patients. Delayed EA was successfully accomplished in 18 of the 19 surviving patients within 5 months, thereby preserving the native esophagus in all surviving infants. There was no difference in anastomotic complications, gastroesophageal reflux, or survival when the delayed group was compared with those who had a primary repair. CONCLUSIONS: Using delayed EA repair, all children with EA and TEF, regardless of gap length, can have their esophagus preserved. The primary cause of mortality was the association of a severe cardiac anomaly with EA and TEF.

Does posterior sagittal anorectoplasty in patients with high imperforate anus provide superior fecal continence?

The posterior sagittal anorectoplasty (PSARP) has become the primary surgical procedure for patients with high imperforate anus. Very few careful long-term follow-up studies have established the superiority of the PSARP procedure over other surgical repair techniques. The authors' goal was to evaluate the operation and to identify the factors associated with improved continence. The medical records, operative reports, and radiographs of 53 patients (46 male, 7 female) who underwent PSARP for high imperforate anus at Children's Hospital and Medical Center (CHMC) between 1982 and 1990 were reviewed retrospectively. Subjective follow-up data were collected by telephone questionnaire, assessing habits indicative of stool continence, and a "fecal continence score" (FCS) was calculated for each patient. A prospective, 7-day diary assessing similar patterns of fecal continence was completed by each patient's family. The mean age of the patients studied was 8.0 years, and the mean follow-up period was 6.8 years. The telephone questionnaire was completed for 48 patients (94%). Toilet training for bowel continence was successful in 20 patients (42%) and occasionally successful in another 20 patients (42%), but 8 patients (16%) had no awareness of impending stool. Forty-five (94%) were physically active, but 16 (33%) reported social problems related to offending odor. The mean fecal continence score for all patients was 3.0 +/- 1.4 (5=excellent, completely continent), which was nearly identical to published scores for the other types of surgical repair. The FCS did not improve with age. The parents' responses to the telephone questionnaire matched the results obtained from the prospectively collected continence diary data. The authors' results for PSARP in patients with high imperforate anus do not differ substantially from those achieved by other repair techniques. Previously cited prognostic factors such as fistula anatomy and sacral anomalies did not appear to alter the functional results in the authors' series. Aggressive postoperative bowel management should be anticipated in all patients who have high imperforate anus and may benefit those who otherwise would achieve less satisfactory continence.

Management of transected pancreas in children.

The pancreas is the fourth most commonly injured intra-abdominal organ in children who sustain blunt abdominal trauma. Appropriate management of the injured pancreas has been controversial. With the advent of the computerized tomography scan, paediatric surgeons have tended to manage pancreatic injuries non-operatively. However, if pseudocysts develop, non-operative management may necessarily entail a long hospital course involving total parenteral nutrition, drainage procedures and attendant morbidity. The critical element in planning therapy is to determine the status of the pancreatic duct. We have recently encountered five children who suffered blunt pancreatic injury where the main pancreatic duct was determined to have been transected. These children underwent spleen preserving distal pancreatectomy with resultant shorter hospital stays and minimal long-term morbidity. We suggest that in children with pancreatic injury where the main pancreatic duct has been transected early operative management rather than non-operative therapy is the procedure of choice. Endoscopic retrograde cholangiopancreatography should be used to determine the status of the pancreatic duct. This modality can be both diagnostic and therapeutic in appropriate circumstances.

Early recognition of neonatal abdominal wall necrotizing fasciitis.

Necrotizing fasciitis (NF) of the abdominal wall occurring in newborns is associated with a 50% mortality rate. Improved survival requires early diagnosis followed by aggressive surgical débridement. During a 10-year period, we treated 7 infants who developed NF. During the same period, 32 infants were admitted with omphalitis that did not progress to NF. The patients with omphalitis and those with NF were compared. Tachycardia, abnormal white blood cell counts, induration, and violaceous skin discoloration were seen exclusively in the NF patients. Polymicrobial infections were documented in 28% of the omphalitis patients and 86% of the NF patients. All omphalitis patients survived, whereas 5 of 7 (71%) NF patients died. Adjuvant hyperbaric oxygen therapy was used for 4 infants with NF, 2 of whom survived (50%). NF is a highly morbid disease, that can be distinguished from other infant abdominal wall infections by the skin changes, white blood cell counts, heart rate, and microbiologic results. Prompt diagnosis of NF improves survival when combined with aggressive surgical débridement.

Necrotizing enterocolitis in term neonates.

Necrotizing enterocolitis (NEC) is usually a disease of premature infants, but occasionally it affects the term neonate. A 5-year review of NEC at Children's Hospital and Medical Center identified the unique features of this disease in the term neonate. Eighty-one patients with NEC were treated between January 1984 and May 1989. Ten full-term neonates with gestational age greater than 38 weeks were identified for study. Charts were reviewed for recognized risk factors, clinical course, surgical intervention, and outcome. Ninety percent had a birth weight greater than or equal to 2.7 kg, and all were above 2.1 kg. NEC developed early in this group, with onset of disease in the first 48 hours of life in 50% of the group and within the first 4 days of life in 90%. The recognized risk factors of asphyxia, hypoglycemia, polycythemia, and respiratory distress were absent in 60%. Seven of 10 patients required exploratory laparotomy, whereas 3 of 10 required only medical treatment. Indications for operation were perforation in three patients, peritonitis in three patients, and mass in one patient. All patients requiring operations had severe colonic disease, with perforation of the colon in five of seven and full-thickness necrosis without perforation in two of seven. Two patients required total abdominal colectomy. Only one patient with perforated meconium ileus and associated NEC had small bowel involvement. This patient was the only mortality of the group. Subsequent intestinal continuity was restored in all surviving patients with no late complications. Two patients required resection of additional NEC strictures prior to reanastomosis. Of the three medically treated patients, none required subsequent operation for colonic stricture. Our experience indicates that the presentation, clinical course, and operative findings in full-term neonates with NEC differ from those encountered in the premature infant with NEC.

Improved survival with primary anastomosis in the low birth weight neonate with esophageal atresia and tracheoesophageal fistula.

Management of esophageal atresia with tracheoesophageal fistula (TEF) has evolved over the past 5 years at Children's Hospital and Medical Center, Seattle, in the premature patient weighing less than 2.5 kg. Traditionally, these low birth weight infants underwent gastrostomy and fistula ligation followed by repair after a period of weight gain. However, since 1980, of the 31 neonates weighing 2.5 kg or less with type-C TEF, a primary repair was performed in 22 within the first five days of life. There were no mortalities and only one subclinical anastomotic leak. All anastomoses were done extrapleurally in a single-layer interrupted fashion. Staged repair was elected for nine neonates. Seven had severe respiratory distress requiring mechanical ventilation and two in the staged group expired prior to repair. The survival for the entire group of 31 neonates was 94%. Primary repair may be performed safely in the premature neonate. The presence of severe pulmonary disease is the critical factor that might necessitate a staged repair.

Maternal serum alpha-fetoprotein is a marker for fetal anomalies in pediatric surgery.

Maternal serum alpha-fetoprotein (MS-AFP) screening has become part of routine obstetric care. Although elevated MS-AFP was originally associated with neural tube defects (NTD), it is also able to detect several fetal anomalies of interest to a pediatric surgeon, ie, ventral abdominal wall defects, intestinal atresias, and sacrococcygeal teratomas. Previously, decreased MS-AFP had only been associated with fetal trisomies, but not surgically correctable lesions. In the present study, we review our recent experience with both elevated and decreased MS-AFP as a marker to detect fetal anomalies of concern to the pediatric surgeon. Forty-one fetal anomalies were associated with 333 pregnancies referred for follow-up after abnormal MS-AFP screening results from November 1985 through November 1986. One hundred ninety-six were elevated and 139 were decreased. In most cases, evaluation included counseling, repeat MS-AFP, level II ultrasound, and amniocentesis. This revealed elevated MS-AFP to be associated with 32 (16.3%) anomalies (2 NTD, 5 anencephalics, 5 ventral abdominal wall defects, 1 stage IV-S neuroblastoma, 1 renal anomaly, 1 ventriculomegaly, 15 fetal demises, and 2 fetal-maternal bleeds). Decrease in MS-AFP was associated with nine (6.4%) anomalies (2 congenital diaphragmatic hernias, 3 Down's syndrome, 1 Turner's syndrome, 2 duodenal atresias, and 1 choroid plexus cyst). In this study, MS-AFP detected several fetal anomalies known to be associated with abnormal MS-AFP and three anomalies not previously described (congenital diaphragmatic hernia, neuroblastoma, and choroid plexus cyst). Elevated as well as decreased MS-AFP are significant and should be pursued by a full prenatal evaluation.(ABSTRACT TRUNCATED AT 250 WORDS)

Radiotherapy for locally recurrent infantile pancreatic carcinoma (pancreatoblastoma).

The authors report a case of recurrent pancreatoblastoma in a 3-year-old girl who was successfully treated with radiotherapy. The patient had two local tumor recurrences before radiation treatment; the second recurrence was managed with radiation alone. Computerized tomography scan at the completion of radiotherapy showed dramatic tumor regression. Six weeks after radiotherapy was finished exploratory laparotomy was performed; multiple biopsies showed no evidence of tumor. The patient is currently disease-free 2 years after completion of radiotherapy. This case suggests a role for radiation therapy in the management of children with pancreatoblastoma.

Surgical management of the infant with mesenchymal hamartoma of the chest wall.

Mesenchymal hamartoma of the chest wall is a rare lesion that presents as a chest wall mass. Four infants are reported, three of whom underwent extensive chest wall resection.

Surgical management of multiple intestinal atresias.

The unusual problem of multiple intestinal atresias associated with jejunal atresia has been reviewed and seven cases have been summarized. Maintaining maximal bowel length without jeopardizing the patient is of utmost importance. The shish kebob technique has been illustrated in a patient with 15 obstructions. The other cases include one patient with the apple peel deformity and two with atresias associated with gastroschisis. Although these children are small in general, they are bright and otherwise healthy. The 90 percent mortality reported previously has been reversed by a combination of techniques and lessons learned from the leaders in pediatric surgery.

Surgical management of life-threatening and disfiguring sequelae of fulminant meningococcemia.

In recent years, because of prompt diagnosis and effective, aggressive resuscitation, the majority of infants and children with fulminant meningococcemia are surviving. From 1974 through 1984, 135 patients with this diagnosis were treated, and 126 of them survived. Although a purpuric skin rash developed in almost all of these patients initially, in eight of them it progressed to multiple confluent areas of cutaneous gangrene, usually associated with extensive necrosis of underlying subcutaneous fat, fascia, skeletal muscle, and even bone. Tissue necrosis seemed to be most extensive in regions of reduced blood flow, such as the extremities, but it almost never followed a pattern of anatomic vascular distribution. A most significant microscopic finding was the presence of multiple fibrin thrombi in vessels, often in close proximity with the foci of tissue necrosis. Five children who ranged in age from 6 months to 12 years required operation. Initially, all surface wounds were treated like full-thickness burns with silver sulfadiazene (Silvadene) dressings. Once the patient's condition had stabilized and the extent of tissue necrosis was apparent, all necrotic tissue was excised and the resulting wounds were temporarily covered with biologic dressings to assure a clean, viable base for skin grafting. Because the resulting quality of life postoperatively in all five surviving patients has been satisfactory, we recommend an aggressive surgical approach in patients with fulminating meningococcemia, despite what may initially appear to be devastating and even lethal complications of this disease.

The advantages of hemodilution anesthesia for major liver resection in children.

Since 1977, eight children have had major liver resections with the aid of normovolemic hemodilution anesthesia. Five children were 7 months of age or less; the other three were 3 1/2, 8, and 12 years old. This includes three operations for hepatoblastoma, three for hepatoma, and two for mesenchymal hamartoma. There were three right lobectomies, two trisegmentectomies, two extended left lobectomies, and one standard left lobectomy. With the use of this method, operative blood loss has been greatly reduced and operating technique significantly improved. After abdominal exploration and initial liver mobilization, whole blood is removed from the patient and simultaneously replaced with three times the volume of Ringer's lactate solution to maintain adequate intravascular volume. After the major blood loss has ceased, the patient is reinfused with his own blood and a simultaneous diuresis is induced with LASIX to remove the previously administered crystalloid solution. Metabolic demand is reduced by allowing the patient's temperature to drift down to 32 degrees C. Blood loss is further reduced by a Halothane-induced hypotension to a mean arterial pressure of 40 to 50 torr. The diluted blood lost during surgery has a low red blood cell volume per cc; therefore, each cc of blood lost depletes the total red cell volume by a lesser amount. This results in a nearly bloodless operative field which facilitates the ease, speed, and safety of the dissection. Use of this technique can avoid the need for massive intra-operative transfusions and the threat of cardiac arrest due to extensive blood loss during major liver resection in children.(ABSTRACT TRUNCATED AT 250 WORDS)

The acute abdomen in the immunologically compromised child.

From 1976 through 1982, 35 children with leukemia (27), aplastic anemia (4), and solid tumors (4), were evaluated for symptoms and signs of acute abdominal disease. Twenty-six patients required 34 operations, 14 of them for life-threatening events. Sixteen patients received a bone marrow transplant. The most common surgical emergencies were for acute massive Gl hemorrhage (10), biliary tract disease (4), and typhlitis (3). Thirteen (37%) of the children are alive and free of disease, 22 (63%) have died, 13 of them as a direct result of the abdominal complication which occurred. Alterations in the immune system and hematopoietic system caused by the malignant disease and its treatment with chemotherapy and irradiation appears to be responsible for a number of abdominal complications which the surgeon is asked to see. When symptoms and signs of an acute abdomen develop in patients with leukemia or solid tumors, prompt, thorough evaluation and early aggressive surgical treatment is needed if the patient is to survive. The surgeon should be aware of the unusual array of abdominal complications which can occur in this population of patients.

The usefulness of percutaneous fine-needle aspiration biopsy in infants and children.

During the past 2 years percutaneous fine-needle aspiration biopsy has been employed to help establish or confirm diagnosis in 32 infants and children. A 22-gauge needle is used to aspirate the site of suspected disease. For lesions inside the chest or abdomen, the needle is guided with the help of fluoroscopy, ultrasound, or computed tomography (CT) scan. Nineteen of the 32 patients had malignant disease, including lymphoma, neuroblastoma, rhabdomyosarcoma, Ewing's sarcoma, osteosarcoma, and leukemia. In all patients with solid tumors, tissue obtained at operation confirmed the accuracy of the diagnosis. In seven children with suspiciously enlarged lymph nodes, an open biopsy was avoided when the needle aspirate was clearly benign. In four children, the early appearance of metastatic or recurrent malignancy was confirmed without the need for open biopsy. In this small series, there were no false-negative or false-positive needle aspirates, and no complications directly related to the procedure. The skill and experience of the cytopathologist is essential to the success of this technique. Percutaneous fine-needle aspiration biopsy is a safe and reliable alternative method of establishing a diagnosis in infants and children with suspected malignant disease.